Hemolytic Anemias Quiz

False (B)

Membrane skeleton proteins

<p>sickle</p> Signup and view all the answers

<p>Hereditary Spherocytosis = Defect in membrane skeleton proteins Sickle Cell Anemia = Abnormal globin synthesis Thalassemia = Deficient globin synthesis Immuno-hemolytic Anemia = Antibody mediated hemolysis</p> Signup and view all the answers

<p>Splenomegaly (D)</p> Signup and view all the answers

<p>False (B)</p> Signup and view all the answers

<p>Loss of cell membrane relative to cell volume</p> Signup and view all the answers

<p>Plasmodium falciparum malaria</p> Signup and view all the answers

<p>HbS = Substitution of valine for glutamic acid Normal hemoglobin = Tetramer composed of two pairs of similar chains Epidemiology = Common in regions with malaria Morphology = Sickled RBCs under low oxygen tension</p> Signup and view all the answers

<p>Splenectomy (D)</p> Signup and view all the answers

<p>True (A)</p> Signup and view all the answers

<p>96%</p> Signup and view all the answers

<p>Reduced membrane stability of RBCs (A)</p> Signup and view all the answers

<p>True (A)</p> Signup and view all the answers

<p>autosomal dominant</p> Signup and view all the answers

<p>deformable</p> Signup and view all the answers

<p>Spherocytes = Hereditary Spherocytosis Turbulence by a defective valve = Intravascular Hemolysis Bilirubin-rich gallstones = Extravascular Hemolysis Hemoglobinemia = Intravascular Hemolysis</p> Signup and view all the answers

<p>20 days (B)</p> Signup and view all the answers

<p>LDH (D)</p> Signup and view all the answers

<p>True (A)</p> Signup and view all the answers

<p>False (B)</p> Signup and view all the answers

<p>Polymers formed by HbS molecules on deoxygenation</p> Signup and view all the answers

<p>Splenic sequestration</p> Signup and view all the answers

<p>ankyrin</p> Signup and view all the answers

<p>reversible</p> Signup and view all the answers

<p>Hereditary Spherocytosis (B)</p> Signup and view all the answers

<p>Chronic hemolytic anemia = Reduction in red blood cell lifespan Microvascular obstructions = Ischemic tissue damage due to stuck RBCs Acute chest syndrome = Pulmonary complications from vaso-occlusion Autosplenectomy = Loss of spleen function by adulthood</p> Signup and view all the answers

<p>Infection (D)</p> Signup and view all the answers

<p>True (A)</p> Signup and view all the answers

<p>Point mutation at position 6 causing valine substitution</p> Signup and view all the answers

<p>calcium</p> Signup and view all the answers

<p>They become irreversibly sickled (B)</p> Signup and view all the answers

A condition where red blood cells are spherical instead of biconcave. This shape makes them more fragile and prone to destruction.

A condition where the bone marrow fails to produce enough red blood cells.

The removal of the spleen, often performed to treat hereditary spherocytosis.

The most common type of hemoglobin found in adults, composed of two alpha and two beta globin chains.

A condition where red blood cells contain an abnormal form of hemoglobin called hemoglobin S, which causes the cells to sickle under low oxygen conditions.

The substitution of valine for glutamic acid at the sixth amino acid residue of β-globin, resulting in the formation of hemoglobin S.

The tendency of red blood cells to sickle, which is influenced by factors such as oxygen levels, acidity, and temperature.

The frequency of sickle cell anemia is higher in regions where malaria is prevalent due to a protective effect of HbS against Plasmodium falciparum malaria.

Anemia stemming from blood loss, characterized by normocytic and normochromic red blood cells in acute cases. Chronic blood loss leads to depleted iron stores, resulting in microcytic and hypochromic iron deficiency anemia.

A condition where red blood cells are destroyed prematurely, resulting in a decreased lifespan and increased erythropoiesis. This can be caused by intrinsic defects within the red blood cell itself or extrinsic factors outside the cell.

Hemolytic anemia caused by abnormal globin synthesis, leading to defective hemoglobin. Examples include sickle cell anemia.

Hemolytic anemia triggered by external factors impacting red blood cells, like antibodies or infections. Examples include autoimmune hemolytic anemia.

The production of blood cells outside the bone marrow, often seen in severe hemolytic anemias. This occurs due to the bone marrow's inability to keep up with the increased demand for red blood cell production.

A genetic mutation where glutamic acid is replaced by valine at position 6 in the beta globin chain, leading to the formation of hemoglobin S.

Deoxygenation causes HbS molecules to form long polymers, distorting the red blood cell into a sickle shape.

Repeated sickling episodes damage the red blood cell membrane, leading to dehydration and a shorter lifespan.

Sickle cells lose their deformability and get stuck in small capillaries, leading to microvascular occlusion and ischemic tissue damage.

Painful episodes in sickle cell anemia are often caused by microvascular occlusion in the bone marrow, which can progress to infarction.

Acute chest syndrome is a serious complication of sickle cell anemia, often triggered by pulmonary infections or emboli from infarcted bone marrow.

The frequency of sickle cell anemia is higher in malaria-prone regions because HbS provides partial protection against malaria.

In homozygous sickle cell anemia, all HbA is replaced by HbS, while in heterozygotes, only about half is replaced.

The spleen is often damaged by repeated infarction and autosplenectomy in sickle cell anemia.

A peripheral blood smear can show sickle cells, particularly irreversibly sickled cells, which are characteristic of sickle cell anemia.

A condition where red blood cells are spherical instead of biconcave, resulting in increased fragility and destruction.

The horizontal spectrin protein connects to the vertical transmembrane protein band 3 via linker protein Ankyrin to provide stability and flexibility to the red blood cell membrane.

Mutations in genes for spectrin, band 3, or ankyrin can cause hereditary spherocytosis.

Due to membrane instability in hereditary spherocytosis, red blood cells lose membrane fragments as they circulate, becoming spherical.

Spherocytes are less deformable due to their spherical shape, making them more likely to be trapped and destroyed in the spleen.

Hereditary Spherocytosis is most commonly inherited as an autosomal dominant trait, with a recessive form occurring in about 25% of cases.

Red blood cells in Hereditary Spherocytosis lack the central zone of pallor due to their spherical shape.

The spleen plays a major role in removing damaged or irregular red blood cells from the blood.

Splenectomy, or removal of the spleen, can improve the symptoms of hereditary spherocytosis by reducing the rate of red blood cell destruction.